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Primary intrapulmonary thymoma (PIT), defined as the presence of thymoma tissue in the lung without an accompanying mediastinal component, is uncommon and so offers a diagnostic quandary. We describe the case of PIT in an 81-year-old man.
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RATIONALE: Sarcoidosis is a multisystem granulomatous disease with unknown etiology. It affects mainly the lungs, but it can affect almost any other organ. Nevertheless, pleural involvement with the development of pleural effusion is relatively rare. It is usually mild and responsive to treatment with systemic steroids. Here we present a case of rapidly recurring massive unilateral pleural effusion caused by sarcoidosis that was resistant to systemic steroids. PATIENT CONCERNS: A 55-year-old lady presented with shortness of breath of 2-months duration. No other respiratory symptoms were reported. On physical examination, there were signs of left-sided pleural effusion, splenomegaly, and inguinal lymph nodes. These findings were confirmed by chest x-ray showing massive pleural effusion. Work up of the effusion revealed an exudative effusion with lymphocyte predominance. Pan-computed tomography scan revealed multiple thoracic, abdominal and inguinal lymphadenopathy; additionally, a left-sided pleural effusion and an enlarged spleen; that contained variable hypodense nodular lesions. Positron emission tomography-computed tomography showed intense uptake in the spleen and the lymph nodes. Inguinal lymph node biopsy showed non-necrotizing granulomatous inflammation. Due to suspicion of malignancy, left medical thoracoscopy was done, and biopsy of the parietal pleura showed nonspecific inflammation without evidence of malignancy or tuberculosis. DIAGNOSIS: Sarcoidosis was diagnosed based on the finding of the non-necrotizing granulomatous inflammation with no evidence of malignancy or infection on several microbiological and pathological samples. INTERVENTIONS: The patient was treated with repeated pleural fluid drainage. Steroids failed to prevent pleural effusion recurrence. Surgical left side pleurodesis was eventually performed. OUTCOMES: At more than 1 year follow up, the patient showed no recurrence of pleural effusion or development of any other symptoms. LESSONS: Sarcoidosis may rarely present with massive pleural effusion, as this presentation is rare; it is imperative to rule out other causes of massive pleural effusion. Massive pleural effusion in sarcoidosis may be steroid-resistant. Pleurodesis may have a role in such a scenario.
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Derrame Pleural/etiologia , Sarcoidose/complicações , Sarcoidose/patologia , Biópsia , Drenagem/métodos , Dispneia/etiologia , Exsudatos e Transudatos/citologia , Feminino , Humanos , Linfadenopatia/diagnóstico por imagem , Linfadenopatia/etiologia , Linfadenopatia/patologia , Pessoa de Meia-Idade , Pleura/cirurgia , Derrame Pleural/cirurgia , Pleurodese/métodos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Radiografia Torácica/métodos , Recidiva , Sarcoidose/diagnóstico por imagem , Toracoscopia/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Background: Computed tomography (CT) features of cystic fibrosis (CF) lung disease can be objectively quantified using current CT scoring systems to assess the extent and severity of the disease. The aims of this study were to calculate the Santamaria CT scores in adult patients with CF with the predominant CFTR I1234V genotype, determine its reliability, and correlate these parameters with lung function, microbial colonization, compliance to treatment, and exacerbations. Methodology: This retrospective observational study was conducted on adult patients with CF who were regularly followed up in the adult CF service at Qatar via CT scans that were taken not during an acute exacerbation. CT scans were scored using the Santamaria scoring system. Corresponding spirometry, microbiological data of sputum culture, and relevant clinical data were correlated with individual CT scores. Results: Only 23 of the 31 patients underwent CT when not in an acute exacerbation and were included in the study analysis. A total of 20 (87%) patients had the I1234V genotype. There was good agreement between the two radiologists on the Santamaria CT scores with an intraclass correlation coefficient (ICC) value of 0.991. Bronchiectasis was the most consistent finding, followed by interlobular and intralobular septal thickening. Patients with poor lung function and frequent exacerbations had significantly higher CT scores (p = 0.015). The CT scores of patients colonized with Pseudomonas aeruginosa were higher but nonsignificant (p = 0.20). The mean CT scores were significantly higher in patients who were noncompliant to regular treatment than in those who were compliant (p = 0.012). Conclusion: Santamaria CT scores comprise a reliable scoring system for adult patients with CF and can be used to determine the extent and severity of lung disease. P. aeruginosa colonization causes more structural lung damage than other common colonizing organisms. Noncompliance to treatment has a significant impact on the increasing severity of CF lung disease.
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BACKGROUND The clinical presentation in acromegaly is usually insidious, with headaches or visual disturbances being the most common symptoms. Previous studies have shown higher fibrinogen levels, lower protein C and S activity values, and enhanced platelet function in patients with acromegaly compared to a normal population. Nevertheless, the link between hypercoagulability and acromegaly is often overlooked and rarely reported in the literature. CASE REPORT We report a case of a young man with a massive pulmonary embolism as the initial presentation of acromegaly. Extensive workup excluded other causes of thrombophilia. Furthermore, the diagnosis of acromegaly was confirmed by the patient's clinical features as well as laboratory and radiological testing. A literature review on the link between hypercoagulability and acromegaly was performed. CONCLUSIONS This case report shed light on hypercoagulability as an under-recognized serious complication of acromegaly and paves the road for future studies on this topic.